Congenital hemifacial hyperplasia: an embryologic hypothesis and case report

Congenital hyperplasia is a developmental disorder characterized by marked unilateral asymmetry. The structural disproportions from one side to the other far exceed variations commonly seen in the normal population. Classically, as first described in 1836, the overgrowth of tissue is restricted to one-half of the body, including the extremities. In other cases the hyperplastic enlargement involves segmental areas of the body, such as the hemifacial skeleton and an extremity. A case report is presented, and the clinical characteristics of 192 cases of congenital hyperplasia in the literature are briefly reviewed. Use of the word hyperplasia to describe the condition (rather than hypertrophy) is encouraged. The authors propose a new embryologic hypothesis: asymmetrical development of the neural fold and hyperplasia of the neural crest cells are said to form the basis for the disorder. The rewards, risks, and limitations of reconstructive surgery over a 10-year period are discussed.